Optical Coherence Tomography Angiography and Macular Vessel Density Analysis of Cystoid Macular Edema in Gyrate Atrophy.

To understand the microvascular abnormalities in cystoid macular edema (CME) in gyrate atrophy.Spectral-domain optical coherence tomography (SD-OCT) and OCT angiography (OCTA) were used in four consecutive female patients (eight eyes) with clinically and biochemistry-confirmed cases of gyrate atrophy and associated CME. Foveal avascular zone (FAZ) area and macular vessel density percentage were calculated and compared with normal subjects.The average age was 20 years (range: 13 years to 32 years). The mean refractive error was -6.5 diopters (D) (range: -1.0 D to -11.0 D). The average central macular thickness was 509 μm (range: 291 μm to 750 μm). OCTA showed an enlarged FAZ in the deep capillary plexus (DCP) with presence of hyporeflective cysts in both the superficial and deep capillary layers corresponding to CME. Compared to the normal subjects, the mean FAZ area was enlarged and macular vessel density was reduced in both the superficial capillary plexus and DCP; this was statistically significant (P < .05). En face OCT of the DCPs showed classical hyporeflective honeycomb pattern delineating the structural pattern of CME in the inner plexiform and outer plexiform layer.OCTA helps understand the basic pathophysiologic mechanisms in gyrate atrophy of choroid as well as etiology for CME and macular schisis. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:423-427.].